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Supravalvular Ring Mitral Stenosis Medication

  • Author: Michael D Pettersen, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Feb 11, 2014
 

Medication Summary

Medical therapy for supravalvar mitral ring consists of drugs to control pulmonary venous congestion and cardiac failure. The 2 main categories of drugs used are diuretics to promote excretion of excess water and positive inotropic drugs to improve myocardial function. Medical therapy helps to relieve symptoms of pulmonary edema and congestive heart failure (CHF) but does not correct the underlying anatomic problem of obstruction.

Antibiotics for endocarditis prophylaxis are no longer recommended for most patients with congenital heart disease. Some significant exceptions are noted, including patients who have previously had endocarditis or patients within 6 months of their surgical repair. Current American Heart Association guidelines also recommend subacute bacterial endocarditis (SBE) prophylaxis for patients who have undergone mitral valve replacement.[12] For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

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Diuretics

Class Summary

These agents are useful to remove excess water that accumulates in heart failure and to relieve symptoms associated with pulmonary edema and peripheral edema.

Furosemide (Lasix)

 

Drug of choice (DOC) for rapid relief of pulmonary congestion and edema caused by CHF. Useful for maintenance therapy for CHF in patients with supravalvar mitral ring. Promotes renal excretion of water by inhibiting electrolyte-transport system in ascending limb of loop of Henle. Can increase solute and water excretion, even with declining glomerular filtration rate.

Chlorothiazide (Diuril)

 

Thiazide that increases water excretion by inhibiting reabsorption of sodium chloride in distal renal tubule.

Less potent diuretic than furosemide.

Useful in maintenance therapy of CHF; in severe CHF or refractory edema, thiazides act synergistically with furosemide to promote diuresis.

Hydrochlorothiazide (Esidrix, HydroDIURIL)

 

Thiazide that increases water excretion by inhibiting reabsorption of sodium chloride in distal renal tubule.

Less potent diuretic than furosemide, useful in maintenance therapy of CHF; in severe CHF or refractory edema, thiazides act synergistically with furosemide to promote diuresis.

Spironolactone (Aldactone)

 

Counteracts secondary hyperaldosteronism that occurs in cardiac failure; inhibits sodium absorption in collecting duct and has a potassium-sparing diuretic effect. Used alone, produces relatively mild diuresis; however, it may be used with furosemide for synergistic action in severe CHF.

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Inotropic agents

Class Summary

Positive inotropic agents increase the force of myocardial contraction and are used to treat acute and chronic CHF. Some may also provide vasodilatation, improve myocardial relaxation, or increase or decrease the heart rate (positive or negative chronotropic agents, respectively). These additional properties influence the choice of drug for specific circumstances.

Digoxin (Lanoxin)

 

DOC among inotropic agents. Improves CHF by positive effect on myocardial contraction. Also helps to control fast ventricular rate, especially in atrial arrhythmia. Preparations include elixir 0.05 mg/mL and tabs 0.125 or 0.25 mg.

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Contributor Information and Disclosures
Author

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Julian M Stewart, MD, PhD Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College

Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Autonomic Society, American Physiological Society

Disclosure: Received grant/research funds from Lundbeck Pharmaceuticals for none.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Ira H Gessner, MD Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous author Raghavan Subramanyan, MD, DM, to the original writing and development of this article.

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Parasternal long axis echocardiographic image showing a supra mitral variant of supravalvular mitral stenosis. A discrete membrane is visualized superior to but distinct from the mitral valve. The mitral valve appears otherwise normal.
Parasternal long axis color flow image showing a supra mitral variant of supravalvular mitral stenosis. Turbulence is noted at the level of the supra mitral membrane. In this case, Doppler interrogation revealed only mild obstruction.
Apical 4-chamber echocardiographic image showing an intramitral variant of supravalvular mitral stenosis. A membrane is visualized that is closely adherent to the mitral valve leaflets, restricting leaflet mobility.
Apical 4-chamber color flow echocardiographic image showing an intramitral variant of supravalvular mitral stenosis. Color flow imaging demonstrates severe mitral valve stenosis.
Continuous wave Doppler interrogation of the mitral valve in a patient with supravalvular mitral stenosis demonstrates severe stenosis with a mean gradient of 25 mm Hg.
Simultaneous recording of pressures in the pulmonary artery wedge position (PAW) and the left ventricle (LV) shows a large gradient in diastole across the mitral valve. PAW pressure is markedly elevated.
 
 
 
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